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Xx male syndrome, also known as de la chapelle syndrome or 46,xx testicular disorder of sex development (or 46,xx dsd) is a rare intersex condition in which an individual with a 46,xx karyotype develops a male phenotype [2][3][4][5][6] in 90 percent of these individuals, the syndrome is caused by the father's y chromosome 's sry gene being atypically included in the crossing over of genetic. In human biology, the typical understanding of sex determination is that biological men have xy chromosomes, while biological women have xx chromosomes However, the genetics of sex determination is far more complex than this binary distinction suggests While most biological men do indeed have xy chromosomes, there are rare cases where individuals who appear male—exhibiting typical male. Disease information summary 46,xx testicular disorder of sex development is a condition in which a person with two x chromosomes (which is normally found in females) has a male appearance More specifically, people with this condition have male external genitalia, ranging from normal to ambiguous. Xx male syndrome, also known as de la chapelle syndrome, is a condition where an individual has two x chromosomes, which are typically associated with female genetic sex, but exhibits male external genitalia and internal reproductive structures This syndrome is distinct from klinefelter syndrome, which involves an xxy chromosomal arrangement. A rare disorder where individuals with two x chromosomes develop male characteristics due to sry gene translocation. 46,xx testicular or ovotesticular dsd Serkal syndrome (sex reversion and underdevelopment of kidneys, adrenal glands, and lungs) midas syndrome (a triad of microphthalmia, dermal aplasia, and sclerocornea) What is the prognosis of xx male syndrome?